Neurological Development

SCORING • Circle 0 if independent sitting is acquired before 9 months of age. • Circle 1 if sitting is acquired between the 10th and 12th months. • Circle 2 if sitting is acquired or absent after 12 months. Walking Independently Independent walking occurs when the child can take at least three steps unaided. The average age of acquisition is 13 months. SCORING • Circle 0 if independent walking is acquired before 18 months of age. • Circle 1 if walking is acquired between the 19th and 24th months. • Circle 2 if walking is acquired or absent after 2 years. To a certain extent, falling, or walking with legs wide apart or on tiptoes, is normal until the age of 2 years.These observations are therefore not given a score until that age. Putting a Cube into a Cup (by Imitation) The voluntary release of a 2.5 cm cube into a cup with a 10 to 12 cm opening is associated with the gradual disappearance of the grasping reflex. TECHNIQUE With the child seated, the examiner places a 2.5 cm cube and a cup in front of the child. The examiner then demonstrates the activity. The child drops the cube into the cup; the wrist should be slightly extended and the child should not lean on the cup for support. 34 Neurological Development from Birth to Six Years

Image not available. FIG. 4. FINE MOTOR MILESTONES UNTIL THE AGE OF 2 YEARS The arrows indicate the average age of acquisition, the double lines indicate the acceptable age limit, and the gray areas indicate a moderate delay for the fol- lowing three fine motor skills: putting a cube in a cup (by imitation), grasping a pellet (thumb-index pinch), and building a three-cube tower (by imitation). (Reprinted with permission from C. Amiel-Tison. Neurologie périnatale. Paris: Masson; 1999.) SCORING • Circle 0 if putting a cube in a cup is acquired before 10 months • Circle 1 if this activity is acquired between the 11th and 14th months. • Circle 2 if this activity is acquired or absent after 14 months. Technical Descriptions of Observations and Maneuvers 35

Grasping a Pellet Picking up a pellet with the thumb-index pinch requires proper func- tioning of the corticospinal system. This pinching motion implies dis- sociation of finger movements and opposition of the thumb. TECHNIQUE With the child seated, the examiner places a small 0.5 cm object (such as a raisin) on the table in front of the child. The child grasps the ob- ject using thumb-index finger opposition. Slight flexion of the thumb is observed at the metacarpophalangeal joint, and more pronounced flexion is observed at the interphalangeal joint. The child should use the terminal or subterminal surfaces of the thumb and index finger to grasp the object. SCORING • Circle 0 if grasping a pellet is acquired before 12 months. • Circle 1 if this activity is acquired between the 13th and 15th months. • Circle 2 if this activity is acquired or absent after 15 months. Building a Three-Cube Tower (by Imitation) Building a tower using cubes requires good visual motor skills and ad- equate postural control. TECHNIQUE The child is seated at a table adjusted to his or her size; five 2.5 cm cubes are placed on the table in front of the child. The examiner demonstrates the activity once. The child should stack at least three cubes. 36 Neurological Development from Birth to Six Years

SCORING • Circle 0 if building a tower is acquired before 21 months. • Circle 1 if this activity is acquired between the 22nd and 24th months. • Circle 2 if this activity is acquired or absent after 2 years. Passive Muscle Tone Definition: Resistance to Slow Stretching Examiners use slow passive movements to evaluate muscle tone at rest. In adult neurology, passive tone refers to muscle resistance (apart from the effects of gravity or an articular disease) that the clinician notes when moving a joint at rest. By using their own proprioceptive expe- rience, examiners can determine what is too much or too little resist- ance to motion. Even without the measurement of precise angles, the term hypertonia is used when the resistance felt is too strong, and the term hypotonia when the resistance felt is too weak. It is essential to evaluate the range of passive movement during early childhood because of the spectacular changes in tone that occur with maturation (see below). Consequently, the terms hypertonia and hy- potonia imply that the range of passive movement is either too limited or too wide for age standards. Muscle tone at rest is easy to evaluate in older children and adults, but more difficult to assess in newborns and very young children. Exam- iners must be aware of the maturational phenomena that affect pas- sive tone during early childhood. These physiological changes, which occur very rapidly at first and more slowly later, indicate the need for an age-dependent scoring system (see below). NOTE In assessing passive muscle tone, examiners must keep one eye on the angle they are estimating and the other eye on the infant’s face, so they can stop the maneuver if the infant grimaces. Technical Descriptions of Observations and Maneuvers 37

The results of each maneuver are expressed as the angle between two segments of a limb (e.g., the popliteal angle), or position in relation to an anatomical landmark (e.g., the elbow in relation to the midline for the scarf sign), or as the amount of curvature (e.g., of the trunk). Examiners must do the following: 1. Obtain a state of quiet alertness favorable to the child’s re- laxation. 2. Ensure that the child’s head is in the midline in order to avoid eliciting the asymmetric tonic neck reflex. 3. Control the force applied, and stop stretching when the child’s discomfort becomes noticeable. Maturation Profile of Passive Tone in the Limbs from 0 to 6 Years The development of passive tone in the limbs is illustrated in Figure 5. At first, the stages of maturation are rapid and spectacular. As dis- cussed earlier, at 40 weeks term (or 40 weeks of corrected age in preterm infants), physiological hypertonia of flexor muscles in all four limbs is very strong and under subcorticospinal control. Hence, stretching is very limited (first gray area, labeled “T,” in Fig. 5). During the following months, the descending wave of muscular re- laxation (cephalocaudal) is one indication that the upper neuromotor corticospinal system is taking control over the subcorticospinal sys- tem. This progression results in physiological hypotonia (second gray NOTE Cerebral maturation, a central nervous system phenomenon that de- termines the descending wave of relaxation, is not significantly affected by the lifestyle of the child. 38 Neurological Development from Birth to Six Years

Image not available. FIG. 5. MATURATION OF PASSIVE TONE IN THE LIMBS FROM 0 TO 6 YEARS This progression is illustrated by three maneuvers (scarf sign, popliteal angle, and adductors angle), starting with physiological hypertonia, which is charac- teristic of the full-term infant (gray area, T). In nine months, the rapid matura- tion of the corticospinal system leads to physiological hypotonia (gray area at approximately 1 year of age).After 2 years, resistance to passive manipulation increases with maturation of the musculo-ligamentous system.The Roman nu- merals refer to the number of the examination; the Arabic numerals refer to the age of the child in years. T indicates term (40 weeks gestation). area, between 9 and 18 months, in Fig. 5). This hypotonia can vary in intensity: there may be a complete lack of resistance to stretching. The age of onset can also vary, usually from 6 to 9 months. This hy- potonia may persist until the age of 18 months. Thus the sequence from extreme hypertonia to extreme hypotonia, extending over a pe- riod of approximately nine months, is linked to cerebral maturation. Later, between 2 and 6 years of age and up, there is a very slow, pro- gressive increase in resistance to passive stretching. This phenomenon Technical Descriptions of Observations and Maneuvers 39

depends on extracerebral factors. It is linked to the development of muscle mass and the strengthening of articular ligaments and, for this reason, is fairly dependent on the physical activity of the child. A very physically active child will have a musculo-ligamentous system that is more resistant to passive stretching. In order to include passive tone assessment in the neurological exam- ination of infants and young children, the examiner must do the fol- lowing: 1. Modify the normal reference values at each three-month in- terval over the first nine months of life, and at less frequent in- tervals thereafter. 2. Broaden the definition of normalcy (which is indicated by a score of 0) so that individual, familial, and ethnic variations are taken into account. 3. Keep in mind that a complete lack of resistance to stretch- ing is normal between 9 and 18 months and thus is not given a score. 4. Know how to recognize the abnormality referred to as be- nign congenital hypotonia, in which resistance to stretching is very weak from the first few months of life and remains that way.The family’s medical history will reveal hyperextensibility in the as- cendants or siblings and will confirm the absence of neurolog- ical or muscular disorders, thus denoting an isolated charac- teristic. NOTE Independent walking is acquired during the period of physiological hy- potonia. Subsequent maturation of the body (muscles and joints) is vari- able and depends on the child’s physical activity.The progressive increase in resistance to passive stretching is therefore a peripheral phenome- non (due to changes in the musculoskeletal system). 40 Neurological Development from Birth to Six Years

Rapid Stretching and Stretch Reflex Examination: Definition of Spasticity Like the slow stretching described above, rapid stretching is used to identify a deficit in upper motor control. When the entire neuromo- tor and muscular system is intact, passive rapid stretching of one seg- ment of a limb should not cause an increase in resistance. The range of motion is comparable to that obtained with slow manipulation. When control is altered, the response to rapid manipulation will change; this is referred to as spasticity. Two abnormal responses en- able examiners to differentiate between two degrees of severity: the first response (phasic) is brisk and of short duration; the second (tonic) is more marked and protracted (see below). Without providing an extensive pathophysiological explanation of spasticity, we can summarize it as follows: lack of upper control of the myotatic reflex constitutes the main abnormality, causing spasticity, hyperreflexia, and clonus (i.e., rhythmic movements of a segment of a limb, set off by a rapid stretching). The intensity of these three signs is usually similar, but small, unexplained variations can be observed. Spasticity is prevalent in the flexor muscles of the upper limbs and in the antigravity extensor muscles of the lower limbs. The presence of all three signs suggests a lesion in either the corticospinal system or the motor cortical areas. Adductors Angle TECHNIQUE With the child supine, the examiner extends the child’s legs and gen- tly spreads them as far apart as possible. The angle formed by the two NOTE During rapid stretching, muscles become stiff unless higher cortical con- trol constantly modulates the action of spinal stretch reflexes. Technical Descriptions of Observations and Maneuvers 41

Image not available. FIG. 6. ADDUCTORS ANGLE By placing their index fingers parallel to the femoral diaphysis, examiners can easily evaluate the maximum width of the adductors angle (approximately 100° in this diagram). legs is the adductors angle. The extensibility of the left and right ad- ductor muscles (evaluated simultaneously) is measured by the degree of this slow angle (Fig. 6). PATTERN OF CHANGES The adductors angle increases progressively over the first nine months. At 2 months of age, it is 40° to 80°; at 9 months, 100° to 140° or more, with the lower limbs offering very little resistance. Between 9 and 18 months, the age of physiological hypotonia, the angle can be limitless. Over the following years, the adductors angle slowly de- creases until it reaches average adult measurements. 42 Neurological Development from Birth to Six Years

SCORING Scoring is age-dependent. (See the examination chart to record a score of 0, 1, or 2.) Adductors angles that fall below age-standard meas- urements help examiners identify abnormal hypertonia of the adduc- tor muscles. A complete lack of resistance between 9 and 18 months should be recorded with an “X.” If it is detected before 9 months or after 18 months, examiners should record a score of 2. The adductors angle can be asymmetric, even when the total angle is within the normal range.This information is covered in the examination chart under “Comparison of the R and L Sides of the Body.” Popliteal Angle TECHNIQUE While keeping the child’s pelvis on the table, the examiner laterally flexes both of the child’s thighs at the pelvis to each side of the ab- domen. With the thighs held in this position, the legs are extended as much as possible. The angle formed by the legs and the thighs is the popliteal angle. Both the left and right angles are evaluated at the same time. The measurement of this angle indicates the extensibility of the hamstring muscles (Fig. 7). PATTERN OF CHANGES The popliteal angle gradually increases over the first nine months. At 2 months of age it is approximately 90°; at 9 months, 120° to 150°. It can be limitless between 9 and 18 months (the age of physiological hypotonia). The popliteal angle slowly decreases over the following years until it reaches average adult values. Technical Descriptions of Observations and Maneuvers 43

Image not available. FIG. 7. POPLITEAL ANGLE With the child’s knees held on each side of the abdomen, the examiner extends both of the child’s legs as far as possible.The examiner visually evaluates the angle formed between the thigh and the leg (both left and right angles are 90° in this diagram). SCORING Scoring is age-dependent. (See the examination chart to record a score of 0, 1, or 2.) Popliteal angles that fall below age-standard measure- ments help examiners identify abnormal hypertonia of the hamstring muscles. A complete lack of resistance is recorded with an “X” be- tween 9 and 18 months, because the existence of physiological hypo- tonia is often quite marked; it should be given a score of 2 before 9 months and after 18 months. Dorsiflexion Angle of the Foot: Slow Maneuver The dorsiflexion angle as measured by the slow maneuver is also called the slow angle. It enables examiners to measure resistance of the rest- ing triceps surae to slow stretching (gray area in Fig. 8). 44 Neurological Development from Birth to Six Years

Image not available. FIG. 8. DORSIFLEXION OF THE FOOT: NORMAL RESPONSE Dorsiflexion of the foot is first performed slowly (dotted line), then rapidly (solid line). The angles obtained by both manipulations should be identical and within the normal range (white area, 80° or less). TECHNIQUE The examiner flexes the child’s foot toward the leg by slowly pressing the plantar surface. The angle formed by the longitudinal axes of the foot and the leg is the dorsiflexion angle. This maneuver is performed on each foot in turn, with the examiner’s hand placed on the knee to keep the leg straight. (This slow angle measures the extensibility of the triceps surae with the knee extended in order to include the gastroc- nemius.) The slow angle is the smallest angle obtained by applying light pressure. When the child is older, the whole foot, including the heel, is placed in the palm of the examiner’s hand to obtain the max- imum stretching of the triceps surae. PATTERN OF CHANGES Development in the first few months: The starting point varies ac- cording to gestational age at birth. The dorsiflexion angle of the foot is close to 0° in the full-term infant, because the in utero pressure dur- ing the last weeks of pregnancy gradually decreases this angle. In the preterm infant who has reached the age of 40 weeks, the angle remains wide, as it was at birth (e.g., 40° to 50° at 28 weeks). Later, during the first few months, the 0° dorsiflexion angle of the full-term infant grad- ually increases to match the angle of the preterm infant. This is why this maneuver is performed only after the fourth month, and only an- Technical Descriptions of Observations and Maneuvers 45

gles above the normal upper limit are taken into account (more than 80°; gray area in Fig. 8). Later development: If permanent dystonia is associated with spastic- ity detected in the triceps surae (see below), a permanent equine de- formity of the foot may result, leading to the shortening of both the muscle and the tendon, thus making the slow angle excessively wide. SCORING • Circle 0 if the dorsiflexion angle is equal to or less than 80°. • Circle 1 if the angle is between 90° and 100°. • Circle 2 if the angle is equal to or greater than 110°. 1. A moderate muscle shortening (90° to 100°) is compatible with normal gait, because only a limited amount of flexion and extension is needed. 2. A 90° angle is common in very physically active children be- tween 2 and 6 years of age, and this should not be considered “neurological” as long as it is an isolated finding. Dorsiflexion Angle of the Foot: Rapid Maneuver The dorsiflexion angle as measured by the rapid maneuver is also called the rapid angle. It enables examiners to evaluate the spasticity of the triceps surae. TECHNIQUE The same dorsiflexion movement is used as in the slow maneuver, but done more rapidly. The repetition of the manipulation often makes an abnormal response more obvious. 46 Neurological Development from Birth to Six Years

SCORING • Circle 0 if the dorsiflexion angle obtained with rapid stretching of the triceps surae is identical to the angle obtained with the slow ma- neuver (Fig. 8). • Circle 1 if a sudden yet brief contraction is noted during the rapid maneuver. The resistance quickly dissipates, allowing the foot to at- tain the position obtained by the slow maneuver. (A few clonic movements often occur with increased resistance.) This response to rapid manipulation is called phasic (Fig. 9). • Circle 2 if the resistance is strong and sustained, if it almost imme- diately prevents rapid manipulation, and if the movement can only be completed slowly. In this case, the abnormal response to rapid manipulation is called tonic. This is the stretch reflex, characteris- tic of the spastic muscle (Fig. 10). 1. The combination of spasticity and shortening of the triceps surae is typical of spastic diplegia (Little’s disease). Muscle short- ening is secondary to permanent dystonia and often associated with spasticity (which is speed-dependent). Permanent dysto- nia of the triceps surae causes a permanent equine deformity of the foot and therefore pulls the triceps surae insertions closer together. 2. For scoring purposes, rapid manipulation is systematically tested only in the triceps surae since moderate spasticity is al- ways distal: if moderate spasticity is absent in the triceps surae, it will also be absent in the other muscles. However, if the stretch reflex is present in the triceps surae, the examination should be extended to include the other muscle groups in the lower limbs in order to analyze spasticity muscle by muscle. Technical Descriptions of Observations and Maneuvers 47

FIG. 9. DORSIFLEXION OF THE FOOT: Image not available. PHASIC STRETCH The slow angle (dotted line) is within the normal range (80° or less).The rapid angle (solid line) is identical, but a short stop will be felt with or without clonus shortly after starting the maneuver.When this contraction relaxes, the rapid maneuver can be completed to the same extent as the slow maneuver. FIG. 10. DORSIFLEXION OF THE FOOT: Image not available. TONIC STRETCH The slow angle (full dotted line) is indicated in the gray area (greater than 80°) and suggests a shortening of the triceps surae. The rapid maneuver (lower solid line) is prevented by a very strong contraction of the triceps surae, and dorsiflexion can be continued only slowly (upper dotted line). Candlestick Posture Shortening of the trapezius can be due to prolonged abnormal postures in intensive care units. It is also maintained by almost exclusive use of the ventral and dorsal decubitus positions without particular at- tention being paid to the upper extremities. Muscular shortening is caused by the medial and external insertions (clavicle, acromion, and the upper ridge of the scapula) being closer to one another. When the child is observed from behind in a sitting or dorsal decubitus position, the arms are seen to be externally rotated and the forearms flexed in such a way that the upper limbs form a two-armed candlestick. Ex- aminers should indicate the presence of this posture by circling “X.” 48 Neurological Development from Birth to Six Years

SCORING • Circle 0 if the candlestick posture is absent. • Circle “X” if the candlestick posture is present. 1. The candlestick posture (shoulder girdle retraction) is often so marked that it creates a deep crease in the posterior aspect of the arm below the deltoid insertion, resembling a deep gash made by an axe. 2. This posture is abnormal because it results in inactivity of the upper limbs during the critical phase of development when the hands normally join at the midline to manipulate objects. 3. If this posture has been allowed to develop, it can be cor- rected with physical therapy and by placing the child in appro- priate positions during sleep.The candlestick posture is not due to a central neurological disorder, but it should be noted be- cause it prevents a proper neurological examination and im- pedes the development of manipulative skills. Hand and Finger Movements The posture and spontaneous movements of the fingers can be ob- served from the first neurological examination (Fig. 11). Image not available. FIG. 11. HAND AND FINGER MOVEMENTS AND POSTURES Shown from left to right: fine finger movements; closed hand, but can be opened easily; adducted thumb held inside tight fist. Technical Descriptions of Observations and Maneuvers 49

SCORING • Circle 0 if an open hand and fine finger movements are observed; this activity is normal when the infant is awake. • Circle 1 if the hands are constantly closed with no visible finger movements at examination I, conducted between the first and third months, even if the fingers can be spread apart easily. • Circle 2 if the hands are constantly closed from examination II (fourth to sixth months) onward; and circle 2 at any age if no movement of the thumb is observed and if it remains permanently adducted inside a fisted hand. 1. Abduction of the thumb is a pyramidal function, and inactiv- ity of the thumb is a sign of problems with higher cortical con- trol.When the examiner opens the infant’s hand, this posture with the thumb flexed over the palm (shown in Fig. 11) is called adducted or cortical thumb. 2. Prolonged inactivity results in atrophy (or underdevelop- ment) of the thenar eminence and in musculo-ligamentous con- tracture.This must be treated. 3. When thumb adduction is observed from birth, it is highly indicative of a prenatal cerebral lesion. The greater the con- tracture and atrophy, the older is the lesion. NOTE The speed of independent finger movements develops over the first years with the maturation of upper motor control. An intact corti- cospinal tract is necessary to play the piano. 50 Neurological Development from Birth to Six Years

Scarf Sign Maneuver This maneuver evaluates the extensibility of the trapezius, the abduc- tors, and the external rotators of the shoulder (the neck is maintained while the acromial insertion of the trapezius is moved). TECHNIQUE For infants in the first few months of life, this maneuver is performed as follows. With his or her elbow resting on the examination table, the examiner supports the head and neck of the infant in a semi-supine po- sition with one hand. One of the infant’s hands is taken and the arm is pulled across to the opposite shoulder as far as possible. The exam- iner then observes the position of the elbow in relation to the midline. Later, when the infant can sit independently, the maneuver can be per- formed with the child in the sitting position, with the observer either behind or in front of the child. At any age, the infant’s head must be kept in the midline. RESULTS To help examiners with their observations, three elbow positions are illustrated in Figure 12. • Position 1: elbow does not reach the midline • Position 2: elbow passes the midline • Position 3: elbow reaches very far across with little resistance Image not available. FIG. 12. SCARF SIGN Position 1: the elbow does not reach the midline; position 2: the elbow passes the midline; position 3: the elbow reaches well past the midline. Technical Descriptions of Observations and Maneuvers 51

If the examiner feels no resistance (NR), this is also noted on the ex- amination chart. SCORING Scoring is age-dependent. (See the examination chart to record a score of 0, 1, or 2.) At term, resistance to stretching is very strong and then gradually dissipates. At 2 months, the elbow almost reaches the mid- line (this is the first indication of the descending wave of relaxation when upper control is intact). At or before 9 months, there is little or no resistance and the arm can be wrapped around the neck. Examin- ers should give a score for complete lack of resistance only before 9 months or after 18 months of age, not for any age in between, because hypotonia is physiological at this stage of development (9 to 18 months). Comparison of the Right and Left Sides of the Body: Asymmetry within the Normal Range As explained above, definition of a wide normal range of passive tone in the limbs is necessary because of individual variations. Asymmet- ric findings noted during the examination will become evident when comparing the scores of the left and right sides of the body. The ex- aminer should note the following points, however. 1. Asymmetry can be present even when all values (both left and right) have been given a score of 0. This asymmetry represents an abnor- mality in itself, which is clearly visible and significant within the normal range. It is easier to detect a moderate abnormality when it is unilaterally predominant. 2. Asymmetry can be an important orientation marker for a particu- lar type of cerebral lesion, and therefore the examiner may use in- formation as a diagnostic clue. 52 Neurological Development from Birth to Six Years

SCORING • Circle 0 if asymmetry is absent or cannot be categorized. • Circle 1 if one side (left or right) is more tonic than the other. It is possible to confuse asymmetry originating in the central nervous system (CNS) with asymmetry in the musculoskeletal system due to prolonged abnormal postures. Multiple births and prolonged intensive care are two instances in which positional asymmetry is particularly likely. Passive Extension of the Body Axis (Dorsal Curvature) Slow dorsal extension of the trunk evaluates the extensibility of the anterior axial muscles (all the prerachidian and abdominal muscles). Image not available. FIG. 13. DORSAL EXTENSION OF THE BODY AXIS This is evaluated with the child in the lateral decubitus position, with one of the examiner’s hands maintaining the lumbar region. Dorsal extension can be absent or minimal (above), moderate (middle), or excessive (below). Technical Descriptions of Observations and Maneuvers 53

TECHNIQUE With the infant lying on his or her side, the examiner maintains the lumbar region with one hand and pulls the lower limbs backward with the other hand (Fig. 13). SCORING • Circle 0 if dorsal curvature is absent, minimal, or moderate. • Circle 2 if dorsal curvature is excessive, resulting in arching (also called opisthotonos). Passive Flexion of the Body Axis (Ventral Curvature) Slow ventral flexion of the trunk evaluates the extensibility of the poste- rior axial muscles (trapezius and all the paravertebral extensor muscles). TECHNIQUE With the infant lying on his or her back, the examiner grasps both the legs and pelvis and pushes them toward the head to test the maximum curvature of the spine (Fig. 14). SCORING • Circle 0 if ventral curvature is moderate but easy to obtain. • Circle 1 if ventral curvature is absent or minimal. • Circle 2 if ventral curvature is unlimited, indicating extreme hypo- tonia. Comparison of Dorsal and Ventral Curvatures of the Body Axis A precise definition of the degree of passive flexion and extension of the trunk is impossible. Curvature is evaluated visually, not measured; 54 Neurological Development from Birth to Six Years

Image not available. FIG. 14. VENTRAL FLEXION OF THE BODY AXIS This is evaluated with the child in the dorsal de- cubitus position.Ventral flexion can be moderate (above), absent or minimal (middle), or unlimited (below). resistance to passive manipulation is felt through the examiner’s hands, not measured. Flexion and extension vary with age, and both values are greater during the period of physiological hypotonia. They also vary with articular and individual factors, as well as poor tolerance for manipulation when the child is in the supine position. It is by com- paring both curvatures that examiners arrive at the best interpretation of passive tone of the axis and its deviations. Comparative scoring at any age should be as follows. SCORING • Circle 0 if flexion is greater than or equal to extension—that is, there is a certain degree of anterior curvature and little or no dorsal ex- tension. (Flexion and extension are given a score of 0.) • Circle 1 if flexion is more limited than extension. (Flexion is scored 1 because it is minimal or absent, and extension is scored 0 or 2.) Technical Descriptions of Observations and Maneuvers 55

This situation indicates a lack of upper control of the antigravity muscles. • Circle 2 if both curvatures are unlimited. (Both are scored 2.) Com- paring the scores of the two maneuvers offers no benefit, and find- ings are interpreted as extreme axial hypotonia (sometimes called rag doll). Diffuse Rigidity Rigidity cannot be evaluated in the same way as hypotonia or hyper- tonia—that is, by measuring muscular extensibility (angles or curva- tures). Rigidity is detected as an abnormal sensation during passive manipulation. Slow manipulation gives the examiner a feeling of in- creased resistance over the whole movement, such as the resistance felt when trying to bend a lead pipe. The detected resistance includes both the agonistic and antagonistic muscles involved in the joint being ma- nipulated. SCORING • Circle 0 if rigidity is absent. • Circle 2 if rigidity is present. NOTE Passive tone of the axis is permanently affected in children who lack higher cortical control. If the cerebral lesion is minor, the imbalance in the flexor and extensor muscles is latent and becomes apparent only when the curvatures of the trunk are compared. 56 Neurological Development from Birth to Six Years

Rigidity is one of the extrapyramidal signs (subcortical tract) and is therefore present in children with CP with very exten- sive lesions. Rigidity is most often diffuse and can be combined with speed-dependent spasticity; it is rarely an isolated occur- rence in the infant. Motor Activity Facial Expression Observation of facial mimicking is part of the neurological examina- tion. Facial expressions are normally greatly diversified and contribute to showing pleasure, pain, and various discomforts. SCORING • Circle 0 if facial expressions appear normal, symmetric, and varied. • Circle 1 if the infant does not show much facial expression, which indicates insufficient facial motility. Drooling Constant drooling is a sign similar to facial expression, but it is too in- significant before the age of 1 year to be recorded as abnormal. SCORING • Circle 0 if drooling is absent during the first year. • Circle 1 if drooling is present during the second year. • Circle 2 if drooling persists significantly after 2 years of age. If this occurs, it is one of a series of severe signs. • Circle “X” if drooling is present during the first year. Technical Descriptions of Observations and Maneuvers 57

Facial Paralysis Unilateral or bilateral facial paralysis is scored, and the affected side is indicated. SCORING • Circle 0 if facial paralysis is absent. • Circle 2 if facial paralysis is present. Fasciculation of the Tongue Fascicular movements of the tongue are abnormal if they occur at rest and on the sides of the tongue. This is an indication of a lesion in the nucleus of the 12th cranial nerve. SCORING • Circle 0 if fascicular tongue movements are absent. • Circle 2 if fascicular tongue movements are present. Fascicular movements observed only during crying or only in the central part of the tongue are insignificant and are not given a score. NOTE A common misdiagnosis must be noted here. Congenital hypoplasia of the depressor anguli oris muscle (autosomal dominant), resulting in a de- formation of the mouth, is visible only when the child cries and is not to be confused with facial paralysis. 58 Neurological Development from Birth to Six Years

Spontaneous Limb Movements Spontaneous limb movements are observed in the supine position dur- ing the first few months of life. Later, all gestures are observed. It is dif- ficult to quantify spontaneous motor activity precisely, but a global es- timation can be scored. SCORING • Circle 0 if limb movements are smooth and varied at all ages. • Circle 1 if limb movements are insufficient, uncoordinated, or stereotypical. • Circle 2 if limb movements are barely present or very uncoordi- nated. Involuntary Movements Involuntary movements may appear or increase during the second year. They can be described as rapid, involuntary movements that affect vol- untary movements (as in chorea) or as slow, writhing, involuntary movements that interfere with resting postures (as in athetosis). In- voluntary movements are an indication of extensive lesions in the ex- trapyramidal system and hence are often associated with diffuse rigid- ity. Stereotypical hand movements are very specific to Rett syndrome. SCORING • Circle 0 if involuntary movements are absent. • Circle 2 if involuntary movements are present, and describe the type. Dystonia Relatively constant abnormal posture, known as dystonia, is due to con- tractions of the antagonistic muscles, caused by a lack of higher cortical Technical Descriptions of Observations and Maneuvers 59

control. Dystonia causes the trunk, the neck, or part of a limb to remain fixed in an extreme position, resulting in a major functional problem. SCORING • Circle 0 if dystonia is absent. • Circle 2 if dystonia is present. Deep Tendon and Cutaneous Reflexes Deep Tendon Reflexes A rough quantitative system has been designed to permit consistency between examiners. In fact, detecting left/right asymmetry is more use- ful than precisely quantifying the response itself. As far as possible, deep tendon reflexes are tested when the infant is calm and relaxed. Only bicipital and patellar reflexes are scored. SCORING • Circle 0 if the deep tendon reflex consists of a few movements with average amplitude. • Circle 1 if this reflex is very strong. • Circle 2 if this reflex is accompanied by significant clonus and/or ex- tends to other muscle groups or if there is no response during fa- vorable examination conditions. NOTE The terms spasticity (speed-dependent) and dystonia (abnormal pos- ture) are borrowed from adult neurology and they do not perfectly apply to the clinical description of CP. Central disorganization of neu- romotor function is specific to lesions inflicted on a developing brain. 60 Neurological Development from Birth to Six Years

Plantar (Cutaneous) Reflex The results of plantar reflexes cannot be interpreted during the first year. Results of normal flexion of the big toe after cutaneous stimula- tion of the outer edge of the foot are inconsistent. If stimulation is too strong, it elicits an extensor reflex, considered meaningless at this age. Because standardization of the stimulation does not seem to be possi- ble, the extensor reflex is not scored until the age of 1 and is indicated with an “X” for younger children. SCORING • Circle 0 if flexion of the big toe is observed. • Circle 2 if extension of the big toe is observed in children over 1 year of age (Babinski sign). • Circle “X” if extension of the big toe is observed during the first year. Primitive Reflexes Primitive reflexes are indicators of subcortical cerebral functioning. The presence of primitive reflexes is physiological during the first months of life, indicating the absence of CNS depression and an intact brain stem. Testing a few of these reflexes will suffice; it is not neces- sary to check them all. After the first few months, when cerebral func- tioning is normally under the control of the upper hemispheric struc- tures, the persistence of these same reflexes becomes pathological. However, the time required for these primitive reflexes to disappear varies substantially, and this must be taken into account when scoring. Evaluating primitive reflexes is no longer beneficial after 9 months of age, with the exception of the asymmetric tonic neck reflex—often the only primitive reflex that shows subtle persistence. Technical Descriptions of Observations and Maneuvers 61

If the infant is examined during the second or third month, the Moro, finger grasp, or automatic walking reflexes may already be absent if the child’s development has been particularly ad- vanced and rapid.Therefore, the absence of these three reflexes should not be considered abnormal except in a clinical case of CNS depression. Sucking TECHNIQUE Non-nutritive sucking is easily analyzed by inserting the examiner’s little finger curled downward on the middle part of the tongue. This contact alone stimulates the sucking reflex. Sucking is not a continu- ous phenomenon; it includes bursts of movements separated by rest periods. In the full-term infant, there are usually eight or more suck- ing movements in a burst; the rhythm is rapid and bursts last for five to six seconds. A strong negative pressure is felt (infant sucks the fin- ger) when facial motility is normal, ensuring a good seal of the lips over the finger. SCORING • Circle 0 if characteristics of normal sucking are present (rhythmic movements with adequate negative pressure). • Circle 1 if the number of repetitions and negative pressure are in- sufficient. • Circle 2 if sucking is absent or completely inadequate (owing to lack of closure of the infant’s mouth on the examiner’s finger or weak tongue movements). 62 Neurological Development from Birth to Six Years

Moro Reflex TECHNIQUE With the child lying in a dorsal decubitus position, the examiner gen- tly raises the child a few centimeters off the table by both hands, with the infant’s upper limbs in extension. When the examiner quickly lets go of the child’s hands, the child falls back onto the examination table and the Moro reflex appears. The first observation is abduction of the arms with extension of the forearms (arms open), followed by adduc- tion of the arms and flexion of the forearms (arms embrace). During the first part of the reflex, the infant’s hands open completely. Crying and an anxious expression are part of the response. SCORING • Circle 0 if the Moro reflex is present during the first three months. • Circle 2 if this reflex is present after 6 months of age. • The presence of this reflex is insignificant between 3 and 6 months of age and is indicated with an “X.” Grasping Reflex of the Fingers TECHNIQUE The examiner places his or her index fingers in the palms of the infant’s hands. This palmar stimulation causes strong flexion of the fingers, known as the grasping reflex. This maneuver can evaluate both hands at the same time. SCORING • Circle 0 if the grasping reflex is present during the first three months. • Circle 2 if this reflex is present after 6 months of age. • The presence of this reflex is insignificant between 3 and 6 months of age and is indicated with an “X.” Technical Descriptions of Observations and Maneuvers 63

The examiner will be able to lift the infant once he or she has grasped the examiner’s fingers.This is made possible as the re- sponse spreads to all of the flexor muscles in the upper limbs. Once lifted off the examination table, the infant can support all or part of his or her own weight. If the infant is very alert and participating well in the examination, his or her head will pass through the body axis and the flexed lower limbs will lift up.This maneuver is more spectacular than useful because it is caused by a primitive reflex (grasping) and a stretch reflex of the flexor muscles of the upper limbs, which contract in response to rapid stretching. However, since the harmonious nature of the motor reflex involves the whole body, and since this maneuver can be perfectly performed only when the infant is very alert and in- teracting well with the examiner, this manipulation (called re- sponse to traction) remains a good indicator of the optimal state of the neonate. Automatic Walking Reflex TECHNIQUE In the first few months, the infant is held in a vertical position, the ex- aminer placing one hand in the upper thoracic region, with the thumb and middle finger under each armpit (the index finger is kept free to prevent the head from moving too much, if necessary). After the first few months, the infant is held in a vertical position by placing one hand under each armpit. The examiner observes the straightening of the legs and trunk. The infant should support most of his or her own body weight for a few seconds. The infant is then gently tilted forward and should take a few steps. NOTE Automatic walking implies a rhythmic contraction of the antigravity mus- cles elicited by cutaneous contact on the sole of the foot. Newborns can climb up stairs but cannot climb down stairs. 64 Neurological Development from Birth to Six Years

SCORING • Circle 0 if the automatic walking reflex is present during the first three months. • Circle 2 if this reflex is present after 6 months of age. • The presence of this reflex is insignificant between 3 and 6 months of age and is indicated with an “X.” Asymmetric Tonic Neck Reflex (ATNR) (or Fencing Posture) TECHNIQUE During the first few months, spontaneous asymmetric tonic neck re- flex (ATNR) can be observed when the infant is lying in a dorsal de- cubitus position. If the infant’s head is rotated to either side, a partic- ular posture of the limbs is observed. In its obvious form (Fig. 15), the infant extends the upper limb on the side toward which his or her face is turned (facial side) and flexes the upper limb of the opposite side (oc- cipital side). A similar but less prominent response may also be elicited in the lower limbs. This posture is called evident ATNR (see the ex- amination chart). Like other primitive reflexes, this fencing posture (fa- cial arm extended and occipital arm flexed) is normal in preterm and full-term newborns during the first few months of life. After the age of 6 months, evident ATNR is a sign of insufficient upper control over subcortical cerebral function. It remains present in severe CP and se- riously restricts voluntary motor activity. After 4 years of age, subtle persistence of this posture is evaluated. (Be- fore 4 years, this reflex is too difficult to elicit because the child does not understand the instructions.) The child is placed on hands and knees with arms extended. To avoid the elbows being locked in ex- tension, the child’s hands are turned inward, with fingers facing each other. The examiner passively rotates the child’s head. Normally, this passive rotation does not change the support of the arms and they re- main extended. If flexing of the occipital arm is observed (Fig. 16), Technical Descriptions of Observations and Maneuvers 65

FIG. 15. SPONTANEOUS/EVIDENT Image not available. ASYMMETRIC TONIC NECK REFLEX (ATNR) During the first few months, this reflex is observed with the child in the decubitus dorsal position.The fencing posture (oc- cipital arm flexed, facial arm extended) indicates that passive tone of the limbs is not yet independent of the position of the head. ATNR is present, but it will barely, if at all, interfere with voluntary motor activity. In the chart, a positive result is noted as elicited ATNR. SCORING • Circle 0 if ATNR is absent at 6 months of age. • Circle 2 if ATNR is markedly present after 6 months. NOTE Strong deep tendon reflexes, the Babinski sign, and primitive reflexes are not abnormal in the newborn or during the first few months.The very young infant can be described at this age as “physiologically spastic.” 66 Neurological Development from Birth to Six Years

Image not available. FIG. 16. ELICITED ASYMMETRIC TONIC NECK REFLEX (ATNR) With the infant on all fours (arms extended, head in the axis), the examiner passively rotates the head. If this maneuver causes the occipital arm to flex, the ATNR reflex is still present. • Circle 1 if, after the age of 4 years, flexing of the occipital arm is ob- served when the infant is on all fours (elicited ATNR). Before 6 months of age, the presence or absence of this reflex is in- significant and is indicated with an “X.” Right/Left Asymmetry in Primitive Reflexes Asymmetry in reflex responses can be observed when responses are normally present at the infant’s age (i.e., scored 0). In this case, the ex- aminer should indicate the affected side (sluggish or absent response). Asymmetry is particularly observed in the Moro reflex and the grasp- ing reflex. Technical Descriptions of Observations and Maneuvers 67

Image not available. FIG. 17. LATERAL PROPPING REACTION (WHILE SEATED) When the sitting child receives a brisk push at shoulder level, he or she avoids falling by extending the opposite arm. Postural Reactions Postural reactions appear during the first year in response to rapid move- ments felt by the infant. Once they appear, they persist throughout life. The absence of postural reactions is interpreted according to age. Lateral Propping Reaction While Seated TECHNIQUE With the infant sitting independently, the examiner briskly pushes the child laterally at shoulder level. The child should extend his or her arm to the opposite side to avoid falling (Fig. 17). This postural reaction is elicited only after a solid sitting position is acquired. It is normally present between 6 and 8 months of age, as soon as the child can sit independently, and is therefore not tested during the first six months. 68 Neurological Development from Birth to Six Years

SCORING • Circle 0 if the lateral propping reaction is present from 6 months of age. • Circle 1 if this reaction is incomplete between 9 and 24 months. • Circle 2 if this reaction is absent after 9 months (even if the test can- not be performed because of the child’s inability to sit independ- ently) or if the response is incomplete after 24 months. If the lateral propping reaction is incomplete or absent between 6 and 9 months of age, this cannot be interpreted and the examiner should circle “X.” Parachute Reaction TECHNIQUE While held in a ventral suspension position against the examiner’s body, the child is quickly pushed with the head forward toward the examination table. The infant should rapidly extend the upper limbs with open hands, as if to protect himself or herself from falling (Fig. 18). This reaction appears in a precise form only between 8 and 9 months and is therefore not tested during the first six months. SCORING • Circle 0 if the parachute reaction is present from 6 months of age. • Circle 1 if this reaction is incomplete between 9 and 24 months or absent between 9 and 12 months. NOTE The parachute reaction is very useful in daily life for catching one’s bal- ance, which is why it is dangerous for people to walk with their hands in their pockets. Technical Descriptions of Observations and Maneuvers 69

Image not available. FIG. 18. PARACHUTE REACTION (FORWARD) The child is quickly pushed forward toward the examination table; the child protects himself or herself by extending both arms and opening both hands. • Circle 2 if this reaction is absent after 12 months or incomplete after 24 months. If the parachute reaction is incomplete or absent between 6 and 9 months, this cannot be interpreted and the examiner should circle “X.” Qualitative Abnormalities in Gross Motor Function and Acquired Deformities Certain abnormal signs preceding the acquisition of a motor function are interesting to note since they often provide clues about the most likely pathophysiological mechanism. However, these signs can also be misleading and cause the examiner to wrongly conclude that a motor function has been acquired. The most common examples are analyzed and scored, if necessary, according to age. Only after 2 years of age (examinations VII, VIII, IX, and X) does scoring become more precise. 70 Neurological Development from Birth to Six Years

Image not available. FIG. 19. HEAD HELD BEHIND THE AXIS BY HYPERTONIC OR SHORTENED TRAPEZIUS MUSCLES Head control seems to be present, but the head is not in the axis and the chin points upward. At the nape of the neck, the trapezius muscles feel short and stiff. Head Control Holding the Head behind the Axis. Head control behind the axis com- monly occurs during the first six months. The head does not fall for- ward when the trunk is leaned forward, and the chin points upward in a typical chin-forward position (Fig. 19). The examiner can feel that the trapezius muscles are short and stiff. This false control of the head can be the result of hypertonia of the extensors (a CNS problem) or shortening of the trapezius muscles due to abnormal postures. Because the interpretation is uncertain, head control behind the axis is not scored before 2 years of age. After 2 years and depending on the circumstances, the CNS nature of this sign is easier to establish and is scored on the examination chart. SCORING • Circle 0 if head control behind the axis is absent. • Circle 2 if this abnormality is present. Technical Descriptions of Observations and Maneuvers 71

Before 2 years of age, the presence of this abnormality is indicated with an “X.” Poorly Maintained Head Control Due to Fatigue. Poor head control when fatigued is an indicator of a partial disability affecting both the flexors and the extensors. The child can voluntarily lift his or her head on request, but can support it for only a relatively short time owing to fatigue or lack of attention. This abnormality is not scored before 2 years of age. If still present after 2 years, the significance of this ab- normality is very serious. SCORING • Circle 0 if poor head control when fatigued is absent. • Circle 2 if this abnormality is present. Before 2 years of age, the presence of this abnormality is indicated with an “X.” Sitting Position Failure to Sit Due to Falling Forward. At this most insignificant stage in the acquisition of independent sitting, falling forward is a result of a certain degree of axial hypotonia. It is not scored until 9 months of age (because it is a simple exaggeration of a physiological phenomenon). SCORING • Circle 0 if failure to sit due to falling forward is absent. • Circle 1 if this abnormality is present between 9 and 12 months of age. • Circle 2 if this abnormality is present after 12 months. Between 6 and 9 months of age, the presence of this abnormality is in- dicated with an “X.” 72 Neurological Development from Birth to Six Years

Image not available. FIG. 20. FALLING BACKWARD WHEN SITTING AND EXCESSIVE EXTENSION WHILE STANDING These abnormalities are often linked together and are an indication of a lack of upper control. (The mother indicates that her child cannot maintain a sit- ting position and always wants to stand.) (Reprinted with permission from C.Amiel-Tison and A. Grenier. Neurological Assessment during the FirstYear of Life. New York: Oxford University Press; 1986.) Failure to Sit Due to Falling Backward. Falling backward is caused partly by an inability to adopt the tripod position because hypertonia of the flexor and adductor muscles in the legs keeps the knees too high and close to one another, and partly by unbalanced axial tone in favor of the extensor muscles. Falling backward is therefore unavoidable (Fig. 20). The presence of this abnormality is scored from 6 months of age (because it is not an exaggeration of a physiological phenomenon). SCORING • Circle 0 if failure to sit due to falling backward is absent. • Circle 1 if this abnormality is present between 6 and 12 months of age. • Circle 2 if this abnormality is present after 12 months. Technical Descriptions of Observations and Maneuvers 73

Falling backward is very often observed in cases of spasticity since it is an indicator of insufficient upper control in axial tone. It is often associated with an arching response to standing on the floor (Fig. 20), through the same mechanism. Poorly Maintained Sitting Position Due to Fatigue. Poor maintenance of the sitting position when fatigued has the same significance as that in- dicated for head control when fatigued. It is scored after 2 years of age. SCORING • Circle 0 if poor sitting due to fatigue is absent. • Circle 2 if this abnormality is present after 2 years of age. Before 2 years of age, the presence of this abnormality is indicated with an “X.” Acquired Deformities. Scoliosis is a common complication of CP in cases of unilateral or predominantly unilateral lesions, according to the distribution of paralysis, spasticity, and orthopedic problems in the pelvic region and lower limbs. This deformity is scored after 2 years of age. SCORING • Circle 0 if scoliosis is absent. • Circle 1 if this deformity is present after 2 years of age. Kyphosis is a common deformity caused by spasticity and the short- ening of the hamstring muscles. When in a sitting position, the ischium slides forward to draw the hamstring insertions closer together. Kyphosis worsens when the child is seated on the floor. This deformity is scored after 2 years of age. 74 Neurological Development from Birth to Six Years

SCORING • Circle 0 if kyphosis is absent. • Circle 1 if this deformity is present after 2 years of age. Standing Position Excessive Extension While Standing. Excessive extension while stand- ing is an excessive reaction of the antigravity muscles that creates an opisthotonos (or arched) posture. This is abnormal at any age (Fig. 20). SCORING • Circle 0 if arched posture while standing is absent. • Circle 2 if this deformity is present. Lower Limb Deformities. Scissoring of the lower limbs, crossing of the legs in extension, is most often associated with an equine deformity of the foot, making standing difficult. The presence of scissoring is ab- normal at any age. SCORING • Circle 0 if scissoring of the lower limbs is absent. • Circle 2 if this deformity is present. Scissoring is a strong indicator of spastic diplegia. One symptom of spastic diplegia is spasticity in the adductor muscles of the thigh. However, early shortening of these muscles due to ab- normal postures in the neonatal period most often precedes spasticity. In the absence of immediate attention in the intensive care unit, scissoring is partly a sign of a posture-induced deform- ity and partly a symptom of a CNS disorder due to a lack of upper control. Technical Descriptions of Observations and Maneuvers 75

Some additional lower limb deformities include the following: Permanent flexion of the hip: a limitation of full extension. Permanent flexion of the knee: a limitation of full extension. Equine deformity of the foot: a permanent and irreducible extension of the foot. Dislocation of the hip: a serious complication of spasticity that should be prevented by adapted positioning from birth. The presence of each of these deformities is indicated with an “X.” Only the most common deformities have been mentioned here. Other abnormal standing postures should be clearly described according to dystonic muscle groups and acquired muscular shortenings. Gait Only a clinical analysis of walking (not a laboratory assessment) is in- cluded in the examination chart. Gait analysis requires keen observa- tion for defining specific types of limping. The most common of these abnormalities observed in infants with CP are the following: Spastic gait: linked to spasticity in the antigravity muscles with toe- walking and lifting of the body each time the foot touches the floor (skipping). Ataxic gait: linked to a balance problem with broad-based stance and frequent falls. NOTE With immediate and constant adapted positioning of the child from birth, “classical” spastic diplegia becomes “modern” diplegia or diplegia without scissoring. 76 Neurological Development from Birth to Six Years

Hemiplegic gait: linked to a muscular disability that makes the child circumduct a leg (like a scythe). Walking with assistance (tripod, walker, etc.): this should also be noted. The presence of each of these abnormalities is indicated with an “X.” Technical Descriptions of Observations and Maneuvers 77



Chapter 4 Clinical Profiles According to Age • Step-by-Step Profiles up to 2 Years • Short-Term Profile at 2 Years (Corrected Age) • Annual Profiles up to 6 Years



Step-by-Step Profiles up to 2 Years General Comments The detailed descriptions and scoring instructions for each observa- tion or maneuver of the pediatric neurological examination (as given in Chapter 3) increase objectivity and promote the use of a common language. This analytical approach is the basis of all neurological fol- low-ups. However, using this information is not as simple as it may seem, because the data from three age periods (before, at, and after the age of 2 years) must be treated differently. Before 2 Years of Age. Examiners must be extremely careful when in- terpreting the scoring results of a single examination in children under 2 years of age. As we have seen, individuals vary greatly in muscle tone and motor development. What should examiners do about abnor- malities observed during the first two years of life? Some sound advice is to conduct step-by-step profiles based on groupings, or clusters, of abnormalities. To analyze and define these clusters, examiners must use the scores of 0, 1, and 2 recorded in each section of the examination chart for the first six examinations (I to VI) to gather together the most important signs and symptoms. 1. For each section: Examiners should assign the highest score to the entire section—that is, individual scores for each item are not summed. 2. Overall interpretation of the examination at each age period: Severe deficit is defined by the predominance of a score of 2 based on the total number of sections indicated in the tables in this chapter (Ta- bles 1 to 4). Moderate deficit is defined by the predominance of a score of 1 based on the total number of sections indicated in each table. Note that a few scores of 2 are acceptable for the moderate deficit category. These profiles serve as provisional conclusions for medical records, al- lowing precise indications for possible interventions. The profiles Clinical Profiles According to Age 81

should not be used in any other way for children under 2 years of age. This is why the profiles described in the tables are not incorporated in the examination chart. Any system based on calculation of an over- all score would result in distorted interpretations of neurological or maturative events. The purpose of analyzing these successive abnor- mal profiles is to identify a trend rather than a cut-off point. Only the most frequent and typical clinical profiles are described here. The following criteria are taken into account for each age period: 1. The nature of the observed neuromotor abnormalities, their group- ing, and their functional consequences. 2. The fixed nature or progressive trend of the observed signs (de- scribed as static or dynamic). 3. The emergence of specific neuromotor signs with cerebral maturation. 4. Concomitant changes in brain growth, as evidenced by measure- ment of head circumference (HC) growth and examination of the fontanels and cranial sutures. 5. The detection of related sensory or cognitive deviations or epilepsy. During the first nine months, observed signs are still nonspecific. In other words, with the exception of extensive and severe brain damage, this is a “wait and see” period. Great care must be exercised in dis- cussions with parents during this time. At approximately 9 months of age, neuromotor abnormalities become specific and related sensory or cognitive deviations become progres- sively more evident. So as to remain simple in our descriptions of some of the most typical profiles, we assume that prevention of secondary muscular shortening by adapted positioning has been successfully applied from the first day of life and pursued thereafter. In other words, the current results of the neuromotor examination are not, or at least are only slightly, con- taminated by secondary orthopedic consequences of the CNS insult (particularly for the trapezius in the upper limbs and adductor mus- cles in the lower limbs). 82 Neurological Development from Birth to Six Years

At 2 Years (Corrected Age). At 2 years of age the situation becomes clearer. Children with neurological signs who have not acquired the ability to walk independently are diagnosed as having cerebral palsy (CP). A symptomatic and topographical diagnosis, as well as an etio- logical diagnosis (if possible), allows the analysis of short-term results for a cohort of children. After 2 Years of Age. Annual profiles allow further clarification about the severity of neuromotor dysfunction in children with CP and an evaluation of related abnormalities of cerebral functions other than neuromotor function. Classification for children 2 years of age and the annual profiles are found on page 18 of the examination chart. Follow-ups should be con- tinued until the age of learning disabilities for children who show per- sisting neurological abnormalities even though they have not been di- agnosed as having CP (because they walked before the age of 2 years). These children are also included on page 18 of the chart. First Trimester—Neonatal Period and First Three Months of Life At the end of the first three months of life, a provisional profile per- mits the clinician to determine two clinical characteristics: severe deficit and moderate deficit. The characteristics of severe and moder- ate deficits are summarized in Table 1. If these children could be ex- amined repeatedly throughout the first few weeks and months of life, another clinical characteristic could be determined: static or dynamic profile. Static Profile. A static neurological profile of CP is defined by little ob- servable change during the first weeks after birth. Signs already pres- ent at birth change very little during the following weeks, suggesting the damage occurred before birth (Fig. 21). Certain signs present from the first few days of life—including cortical thumb, high-arched palate, and overlapping cranial sutures (Fig. 22)—serve as strong indicators that the insult is of prenatal origin. It is very important that these signs Clinical Profiles According to Age 83